The association of perineal lipomas with anorectal malformations in the newborn has been reported previously (Shaul DB, Monforte HL, Pena A, et al. Surgical management of perineal masses in patients with anorectal malformations. J Pediatr Surg 2005;40:188-191; Wester T, Rintala RJ. Perineal lipomas associated with anorectal malformations. Pediatr Surg Int 2006;22:979-981). Only 2 cases of accessory labioscrotal fold with perineal lipoma have been reported (Redman JF, Ick KA, North PE. Perineal lipoma and an accessory labial fold in a female neonate.